Renal Pathology I Flashcards
What is the hallmark of nephritic syndrome?
Hematuria of glomerular origin
What are the characteristics of nephritic syndrome?
(i) hematuria with dysmorphic cells and red blood cell casts in the urine
(ii) some degree of oliguria and azotemia
(iii) hypertension
What is indicative of glomerular hematuria?
Presence of dysmorphic red cells and red blood cell (RBC) casts indicates glomerular hematuria
What can cause glomerulonephritis?
Glomerulonephritis may be caused by deposition of circulating immune-complexes within glomeruli because of physico-chemical properties and hemodynamic factors peculiar to the glomerulus. The immune complexes elicit an inflammatory reaction in the glomerulus, which is the site of their deposition via complement and leukocyte-mediated pathways.
Acute post infectious glomerulonephritis typical clinical presentation is?
- Children (6-10): acute nephritic syndrome with hematuria, edema, hypertension, renal failure
What is the main organism that can lead to acute post-infections glomerulonephritis?
Group A Streptococcus
What is the pathogenesis of acute post infectious glomerulonephritis?
Post infection, abundant circulating immune complexes with propensity to deposit in the subendothelium develop. It is the activation of complement on the subendothelial and mesangial deposits, which are in contact with the circulation, that drives the influx of inflammatory cells essential for the initiation of glomerulonephritis with its endocapillary proliferation.
What is very useful for diagnosis of post infectious glomerulonephritis?
The large subepithelial deposits, “humps” of immune complex
What are the major lab indications for post infectious glomerulonephritis?
ASO↑ (anti-streptococcal antibody)
C3↓ (decreased complement)
What is the typical immunofluorescene finding in post infectious glomerulonephritis?
The typical pattern is “lumpy-bumpy stain for IgG and C3 in the mesangium and glomerular capillary wall.
What is the appearance of the urine in post infectious glomerulonephritis?
Smoky or tea colored
In this case is kidney biopsy necessary in post infectious glomerulonephritis?
a. No if course is typical
b. Always
c. Not sure
d. Only if course is atypical
e. A and D
e. A and D
What test(s) may be helpful in the differential diagnosis in this of post infection glomerulonephritis?
a. ASO (antistreptolysinOantibody)
b. Complement level
c. both
d. neither
c. both
What is IgA used for?
IgA is the second most common serum immunoglobulin and is the main immunoglobulin in mucosal secretions.
How can IgA be involved in glomerulonephritis?
IgA can be involved in immune complex formation and deposition in tissues and is involved in the pathogenesis of IgA glomerulonephritis.
What is the typical clinical presentation of IgA nephropathy?
IgA nephropathy usually affects children and young adults and begins with episodes of gross hematuria that occur within 1-2 days of a nonspecific upper respiratory tract infection. Typically, hematuria lasts several days and then subsides, to recur every few months.
What is the most common type of glomerulonephritis worldwide?
IgA nephropathy
What are the general features of IgA nephropathy?
- recurrent gross and microscopic hematuria
- episodes of gross hematuria within 1-2 days of a nonspecific upper
- respiratory tract infection (also gastrointestinal or urinary tract)
- hematuria for days, recurrence every few months
What is IgA nephropathy AKA?
Berger Disease
Who is most often affected by IgA nephropathy?
Children and young adults
What is the pathogenesis of IgA nephropathy?
Mucosal infection leads to production of IgA and formation of IgA containing immune complexes which deposit in the mesangium
What are complement levels like in cases of IgA nephropathy?
Normal
Why does IgA nephropathy not affect complement levels?
- IgA is a poor activator of the complement system
- In IgA nephropathy, complement involvement is via the alternative
- Complement levels are replenished by the liver and therefore they remain normal
What is the hematuria in IgA nephropathy like?
Usually painless and follows infection of mucosa like GI/respiratory tract/urinary tract
Is kidney biopsy needed in IgA nephropathy?
Often yes
What finding in the histology is diagnostic of IgA nephropathy?
IgA-containing immune complexes in the mesangium
What is the general cause of hereditary nephritis?
Mutations in glomerular basement membrane proteins
What is the best studied type of hereditary nephritis?
Alport Syndrome
What is the inheritance pattern of most hereditary nephritis’?
X-linked
What is the triad of symptoms with Alport Syndrome?
(i) nephritis
(ii) nerve deafness
(iii) various eye disorders, early cataracts
How do females get Alport Syndrome most commonly?
X chromosome inactivation
What is found in microscopy that is diagnostic for Alport Syndrome?
Lamina densa splitting and lamination,“basket weave”
Are immune complexes seen in Alport Syndrome?
NO
What is the general presentation of post infection glomerunephritis?
Acute nephritic syndrome
Post infection glomerunephritis Epidemiology
Children
Post infection glomerulonephritis Labs
ASO↑
Complement↓
IgA Nephropathy general presentation?
Recurrent hematuria
IgA Nephropathy Epidemiology
Young adults
IgA Nephropathy Labs
Normal
Hereditary Glomerulonephritis general presentation?
Hematuria
Hereditary Glomerulonephritis Epidemiology
Children
Hereditary Glomerulonephritis Labs
Normal
